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Treatment of gastrointestinal disease in systemic sclerosis (scleroderma)

Stephanie A Kaye-Barrett, MD
Christopher P Denton, MD
Section Editor
John S Axford, DSc, MD, FRCP, FRCPCH
Deputy Editor
Monica Ramirez Curtis, MD, MPH


Nearly 90 percent of patients with systemic sclerosis (SSc, scleroderma) have some degree of gastrointestinal (GI) involvement [1,2]. The earliest visceral manifestation to be described is generally esophageal disease, which remains the most common source of GI symptoms in SSc, but any part of the GI tract (mouth to anus) may be involved (table 1).

Clinically significant GI dysfunction occurs in approximately 50 percent, with severe involvement (such as malabsorption and intestinal pseudo-obstruction) being observed in less than 10 percent and portending a poor outcome [3,4]. Approximately 70 percent of patients die within three years of the onset of malabsorption, recurrent pseudo-obstruction, or the requirement for hyperalimentation [4].

The management of GI disease associated with SSc will be discussed here. The remaining issues relating to GI dysfunction, including pathogenesis, pathology, clinical manifestations, and diagnosis, are presented separately. (See "Gastrointestinal manifestations of systemic sclerosis (scleroderma)".)


The general management of gastrointestinal (GI) manifestations is outlined in the table (table 2). The appropriate therapy depends upon the location of involvement and resulting symptoms. It is important to recognize the nuances and subtleties of symptoms, since small differences in presentation may result from entirely different pathogenetic mechanisms. As an example, intermittent diarrhea or constipation is most commonly due to intestinal dysmotility and/or pseudo-obstruction, while persistent diarrhea is likely to be secondary to malabsorption and bacterial overgrowth.


The treatment of oral manifestations of systemic sclerosis is largely supportive. As examples:


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Literature review current through: Sep 2016. | This topic last updated: Jan 18, 2016.
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  1. Turner R, Lipshutz W, Miller W, et al. Esophageal dysfunction in collagen disease. Am J Med Sci 1973; 265:191.
  2. Akesson A, Wollheim FA. Organ manifestations in 100 patients with progressive systemic sclerosis: a comparison between the CREST syndrome and diffuse scleroderma. Br J Rheumatol 1989; 28:281.
  3. Cohen S. The gastrointestinal manifestations of scleroderma: pathogenesis and management. Gastroenterology 1980; 79:155.
  4. Steen VD, Medsger TA Jr. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum 2000; 43:2437.
  5. Hendel L. Hydroxyproline in the oesophageal mucosa of patients with progressive systemic sclerosis during omeprazole-induced healing of reflux oesophagitis. Aliment Pharmacol Ther 1991; 5:471.
  6. Hendel L, Hage E, Hendel J, Stentoft P. Omeprazole in the long-term treatment of severe gastro-oesophageal reflux disease in patients with systemic sclerosis. Aliment Pharmacol Ther 1992; 6:565.
  7. Hendel L, Aggestrup S, Stentoft P. Long-term ranitidine in progressive systemic sclerosis (scleroderma) with gastroesophageal reflux. Scand J Gastroenterol 1986; 21:799.
  8. Orringer MB, Orringer JS, Dabich L, Zarafonetis CJ. Combined Collis gastroplasty--fundoplication operations for scleroderma reflux esophagitis. Surgery 1981; 90:624.
  9. Wipff J, Coriat R, Masciocchi M, et al. Outcomes of Barrett's oesophagus related to systemic sclerosis: a 3-year EULAR Scleroderma Trials and Research prospective follow-up study. Rheumatology (Oxford) 2011; 50:1440.
  10. Kahan A, Chaussade S, Gaudric M, et al. The effect of cisapride on gastro-oesophageal dysfunction in systemic sclerosis: a controlled manometric study. Br J Clin Pharmacol 1991; 31:683.
  11. Horowitz M, Maddern GJ, Maddox A, et al. Effects of cisapride on gastric and esophageal emptying in progressive systemic sclerosis. Gastroenterology 1987; 93:311.
  12. Wysowski DK, Bacsanyi J. Cisapride and fatal arrhythmia. N Engl J Med 1996; 335:290.
  13. Emmanuel AV, Shand AG, Kamm MA. Erythromycin for the treatment of chronic intestinal pseudo-obstruction: description of six cases with a positive response. Aliment Pharmacol Ther 2004; 19:687.
  14. Ghrénassia E, Avouac J, Khanna D, et al. Prevalence, correlates and outcomes of gastric antral vascular ectasia in systemic sclerosis: a EUSTAR case-control study. J Rheumatol 2014; 41:99.
  15. Kaye SA, Lim SG, Taylor M, et al. Small bowel bacterial overgrowth in systemic sclerosis: detection using direct and indirect methods and treatment outcome. Br J Rheumatol 1995; 34:265.
  16. Soudah HC, Hasler WL, Owyang C. Effect of octreotide on intestinal motility and bacterial overgrowth in scleroderma. N Engl J Med 1991; 325:1461.